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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Gerstmann-Straussler-Scheinker disease


Other Names for this Disease
  • Amyloidosis cerebral with spongiform encephalopathy
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Gerstmann Straussler Scheinker syndrome
  • GSSD
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Inheritance


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How is Gerstmann-Straussler-Scheinker disease inherited?

Gerstmann-Straussler-Scheinker disease is inherited in an autosomal dominant fashion, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person inherits the altered gene from one affected parent. In some people, Gerstmann-Straussler-Scheinker disease is caused by a new mutation. Although these individuals most likely do not have an affected parent, they can pass the genetic change to their children.[1]
Last updated: 7/22/2013

References
  1. Prion disease. Genetics Home Reference (GHR). May 2007; http://ghr.nlm.nih.gov/condition=priondisease. Accessed 3/21/2008.